Phacomatosis pigmentovascularis revisited and reclassified

Arch Dermatol. 2005 Mar;141(3):385-8. doi: 10.1001/archderm.141.3.385.


Objective: To provide a new comprehensible and practicable classification by use of descriptive terms to distinguish the various types of phacomatosis pigmentovascularis (PPV), which has previously been classified by numbers and letters that are difficult to memorize.

Study selection: Published case reports on PPV were reassessed.

Data extraction and data synthesis: A critical review revealed that only 3 well-established types of PPV so far exist. To eliminate the cumbersome traditional classification by numbering and lettering, the following new terms are proposed: phacomatosis cesioflammea (blue spots [caesius = bluish gray] and nevus flammeus); phacomatosis spilorosea (nevus spilus coexisting with a pale-pink telangiectatic nevus), and phacomatosis cesiomarmorata (blue spots and cutis marmorata telangiectatica congenita). Phacomatosis cesioflammea is identical with the traditional types IIa and IIb; phacomatosis spilorosea corresponds to types IIIa and IIIb; and phacomatosis cesiomarmorata is a descriptive term for type V. A categorical distinction of cases with and without extracutaneous anomalies seems inappropriate. The traditional type I does not exist, and the extremely rare traditional type IV is now included in the group of unclassifiable forms.

Conclusion: The proposed new classification of PPV by using 3 descriptive terms may be easier to memorize compared with the time-honored grouping of in part not even existing subtypes by numbers and letters.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Biopsy, Needle
  • Female
  • Humans
  • Immunohistochemistry
  • Incidence
  • Infant
  • Infant, Newborn
  • Male
  • Neurocutaneous Syndromes / congenital
  • Neurocutaneous Syndromes / epidemiology*
  • Neurocutaneous Syndromes / pathology*
  • Pigmentation Disorders / classification
  • Pigmentation Disorders / congenital
  • Prognosis
  • Risk Assessment
  • Sex Distribution
  • Survival Rate